Hereditary human complement C3 deficiency due to reduced levels of C3 mRNA (2001)
- Authors:
- Autor USP: ISAAC, LOURDES - ICB
- Unidade: ICB
- Assunto: IMUNOLOGIA
- Language: Inglês
- Abstract: The aim of this work was to study an eight year-old (L.A.S) of consanguineous parents, who presented with recurrent bacterial infections, vasculitis and extremely low levels of serum C3 (0+15'mü'g/mL). Their parents had four other children who died prior age of six monthes due to unspecified infections and a cardiopathy. The classical and alternative pathways hemolytic activities and generation of opsonins and chemotactic factors derived from the activation of the complement system were markedly affected in the proband's serum. In vitro addition of purified C3 restored the classical pathway-dependent hemolytic activity of his serum. Autoradiographs of the proband's LPS-stimulated and 35 S-labelled fibroblast supernatants after SDA-PAGE revealed no C3 'alfa' and 'beta' chains while his mother presented chains of the ecxpected sizes. The amount of C3 mRNA synthesized by the proband's fibroblasts, as evaluated by RT-PCR assays , was greatly reduced. No great insertions or deletions could be observed in the proband's gene by Southern blotting analysis. These data suggest that diminished production or stability of this mRNA may be the cause for this deficieny
- Imprenta:
- Publisher: Comissão de Cultura e Extensão Universitária do ICB/USP
- Publisher place: São Paulo
- Date published: 2001
- Source:
- Título: Resumos
- Conference titles: Congresso do Instituto de Ciências Biomédicas
-
ABNT
ULBRICH, A G et al. Hereditary human complement C3 deficiency due to reduced levels of C3 mRNA. 2001, Anais.. São Paulo: Comissão de Cultura e Extensão Universitária do ICB/USP, 2001. . Acesso em: 02 out. 2024. -
APA
Ulbrich, A. G., Florido, M. P. C., Nudelman, V., Reis, E. S., Baracho, G. V., & Isaac, L. (2001). Hereditary human complement C3 deficiency due to reduced levels of C3 mRNA. In Resumos. São Paulo: Comissão de Cultura e Extensão Universitária do ICB/USP. -
NLM
Ulbrich AG, Florido MPC, Nudelman V, Reis ES, Baracho GV, Isaac L. Hereditary human complement C3 deficiency due to reduced levels of C3 mRNA. Resumos. 2001 ;[citado 2024 out. 02 ] -
Vancouver
Ulbrich AG, Florido MPC, Nudelman V, Reis ES, Baracho GV, Isaac L. Hereditary human complement C3 deficiency due to reduced levels of C3 mRNA. Resumos. 2001 ;[citado 2024 out. 02 ] - Ultrastructural aspects of alpha - 2 - macroglobulin activited mouse macrophages
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